Treatment of gastrointestinal disease in systemic sclerosis (scleroderma)
- Stephanie A Kaye-Barrett, MD
Stephanie A Kaye-Barrett, MD
- Consultant and Honorary Lecturer in Rheumatology
- The Chelsea and Westminster Hospital
- Christopher P Denton, MD
Christopher P Denton, MD
- Professor of Experimental Rheumatology
- Royal Free Hospital, London
- Section Editors
- John S Axford, DSc, MD, FRCP, FRCPCH
John S Axford, DSc, MD, FRCP, FRCPCH
- Section Editor — Scleroderma
- Emeritus Professor of Rheumatology
- St George's University of London
- Nicholas J Talley, MD, PhD
Nicholas J Talley, MD, PhD
- Section Editor — Motility Disorders
- Professor of Medicine, University of Newcastle, Australia
- Adjunct Professor of Medicine and Epidemiology and Consultant, Mayo Clinic, Rochester, MN
- Adjunct Professor, University of North Carolina
- Deputy Editors
- Monica Ramirez Curtis, MD, MPH
Monica Ramirez Curtis, MD, MPH
- Deputy Editor — Rheumatology
- Instructor of Medicine, Part-time
- Harvard Medical School
- Shilpa Grover, MD, MPH, AGAF
Shilpa Grover, MD, MPH, AGAF
- Deputy Editor — Gastroenterology/Hepatology
- Assistant Professor of Medicine, Part-time
- Harvard Medical School
Nearly 90 percent of patients with systemic sclerosis (SSc) have some degree of gastrointestinal (GI) involvement, and approximately one-half are symptomatic [1,2]. Although the esophagus is the most frequently affected part of the GI tract, any part of the GI tract may be involved. Severe involvement occurs in less than 10 percent of patients and is associated with an unfavorable prognosis [3,4]. This topic will review the management of GI disease associated with SSc. Our approach is generally consistent with clinical practice guidelines for the management of common SSc manifestations affecting the GI tract . The pathogenesis, pathology, clinical manifestations, and diagnosis of GI manifestations of SSc are discussed separately. (See "Gastrointestinal manifestations of systemic sclerosis (scleroderma)".)
The management of oral manifestations of systemic sclerosis (SSc) is largely supportive. Regular dental hygiene is necessary to help prevent dental caries. Patients with decreased oral aperture may be able to improve gingival help using adaptive oral hygiene devices (eg, flossers, oscillating-rotating toothbrushes) combined with facial exercises to increase the oral mouth opening [6,7]. Artificial saliva may be used in patients who also have sicca syndrome. (See "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome", section on 'Saliva substitutes'.)
Management of esophageal disease is largely directed toward the amelioration of symptoms of heartburn and dysphagia, which may be due to esophageal dysmotility or gastroesophageal reflux disease (GERD) and its complications (eg, strictures, esophagitis).
Gastroesophageal reflux — The management of GERD in patients with systemic sclerosis (SSc) is similar to that in patients without SSc. However, patients with SSc often have more severe symptoms.
While lifestyle and dietary modification should be recommended in all patients with GERD, these measures are usually inadequate by themselves. These measures include elevation of the head of the bed and selective elimination of dietary triggers (see "Medical management of gastroesophageal reflux disease in adults", section on 'Lifestyle and dietary modification'). Calcium channel blockers and anticholinergic agents, which may be used to treat other manifestations of SSc, can potentially worsen reflux.
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- OROPHARYNGEAL DISEASE
- ESOPHAGEAL DISEASE
- Gastroesophageal reflux
- Esophageal motility disorder
- Infectious and medication-induced esophagitis
- GASTRIC DISEASE
- Gastric antral venous ectasia
- SMALL INTESTINAL DISEASE
- Small intestinal bacterial overgrowth
- Dysmotility and intestinal pseudo-obstruction
- Pneumatosis cystoides intestinalis and pneumoperitoneum
- COLONIC AND ANORECTAL DISEASE
- Fecal incontinence
- LIVER AND BILIARY TREE INVOLVEMENT
- PANCREATIC DISEASE
- SUMMARY AND RECOMMENDATIONS